Myelodysplastic Syndromes

Definition

Myelodysplastic syndromes (MDS) are a group of diseases that involve dysfunction of the bone marrow. Bone marrow is the tissue found within the bones; its task is to create mature blood cells from stem cells. In all forms of MDS, this normal cell-creation process is disrupted by the overproduction of clones of a single stem cell. This leads to a decrease in production of normal red blood cells, white blood cells, and platelets.

The World Health Organization has classified MDS into eight categories. Some forms are more serious than others; all of them are serious enough to require a physician’s care. Thirty percent of people with MDS develop
acute myeloid leukemia
(AML).
Leukemia
is a cancer of the white blood cells and their parent cells. As more is learned about MDS, experts began to see it as a form of cancer.

Thrombocytopenia occurs when there are inadequate levels of platelets in the blood. Platelets stop bleeding by clotting the blood. Signs of thrombocytopenia include:

Bleeding easily, especially from the nose and gums

Bruising easily

Other symptoms may include loss of appetite, weight loss, and feeling tired.

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. The symptoms for MDS can indicate many other conditions. Doctors have to rule out other conditions before diagnosing MDS.

Tests may include the following:

Blood test to check your red and white blood cell counts and platelet counts and to check how the blood cells look.

Bone marrow
biopsy
to check for MDS. A bone marrow biopsy is the removal of a sample of bone marrow for testing.

Your doctor may also order other tests to rule out other conditions.

Treatment

Treatment for MDS depends on your age, other medical conditions, and how serious the disease is. Treatment also depends on how far along the disease has progressed to AML. Often, treatment includes relieving the symptoms of MDS. Talk with your doctor about the best treatment plan for you. You may be referred to a hematologist and an oncologist. A hematologist specializes in blood diseases. An oncologist specializes in cancer. Treatment options include:

Blood Transfusion

If you have a low red blood cell, white blood cell, or platelet count, you may receive a blood transfusion. A blood transfusion is a treatment that involves receiving blood products (red cells, white cells, platelets, clotting factors, plasma, or whole blood) through a vein. The blood components may come from an unrelated donor, from a related donor, or may have been banked in advance by the recipient.

Antibiotics

If you have a low white blood cell count, you may receive antibiotics to fight infection.

Growth Factors

Growth factors help the bone marrow produce blood cells. The following growth factors may be used to treat MDS:

Granulocyte colony-stimulating factors (G-CSF) and granulocyte macrophage colony-stimulating factors (GM-CSF) are growth factors that help the bone marrow produce white blood cells. Pegfilgrastim is a form of G-CSF that is longer acting.

Oprelvekin is a drug that helps the body produce platelets.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be given in many forms including: pill, injection, or via a catheter. The drugs enter the bloodstream and travel through the body killing mostly cancer cells, but also some healthy cells.

Standard Chemotherapy

There are 3 combinations of chemotherapy drugs used to treat MDS. These combinations include:

Cytarabine and idarubicin

Cytarabine and topotecan

Cytarabine and fludarabine

Hypomethylating Agents

Hypomethylating agents are drugs that slow down the growth of cells, and include:

Decitabine

Azacitidine

Immunomodulating Therapy

Immunomodulating drugs change the immune system, and include:

Thalidomide

Lenalidomide

Immunosuppression Agents

Immunosupression agents suppress the immune system, and include:

Anti-thymocyte globulin (ATG)

Cyclosporine

Stem Cell Transplant

The only cure for MDS is a
stem cell transplant
(SCT). A SCT starts with high-dose chemotherapy to kill the bone marrow, and is then followed by an injection of healthy stem cells. The stem cells used can come from the blood or bone marrow.

There are two types of SCT. The less common one is called an autologous SCT, and occurs when the patient is injected with her own cells after high-dose chemotherapy. The more common method is called allogeneic and occurs when a patient gets donor cells after high-dose chemotherapy.

Many doctors will only perform an SCT on a patient that is aged 50 or younger.

Revision Information

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.